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Published on: September 25, 2013
Pediatric hepatocellular carcinoma in a developing country: Is the etiology changing?
Kumar Palaniappan1, Vibhor V Borkar1, Mohamed Safwan1
1Institute of Liver Disease and Transplantation, Global Health City, Chennai, India.
Insights
Pediatric Hepatocellular Carcinoma (HCC) is shifting towards younger children with congenital liver diseases, necessitating liver transplantation. This trend is linked to successful Hepatitis B Virus (HBV) immunization.
Area of Science:
- Pediatric Oncology
- Hepatology
- Transplantation Surgery
Background:
- Hepatocellular Carcinoma (HCC) is a significant pediatric liver malignancy.
- Traditionally, HCC in children is associated with Hepatitis B Virus (HBV) infection, presenting in older children.
- Younger children (<5 years) with HCC typically have underlying congenital or metabolic liver diseases.
Purpose of the Study:
- To analyze the changing demographic and clinical characteristics of pediatric HCC.
- To evaluate the role of liver transplantation in managing pediatric HCC.
- To identify trends in pediatric HCC associated with congenital/metabolic diseases versus HBV.
Main Methods:
- Retrospective review of pediatric HCC cases over a six-year period.
- Analysis of patient demographics, underlying liver conditions, and treatment modalities.
- Comparison of HCC characteristics based on discovery (preoperative vs. incidental on explant).
Main Results:
- Twelve pediatric HCC patients were identified, with a median age of 5.9 years.
- Eleven patients had congenital or metabolic liver diseases; none had HBV.
- Hepatocellular Carcinoma (HCC) was incidentally discovered in 66.7% of explants, showing less aggressive features. All patients underwent liver transplantation with no recurrence observed.
Conclusions:
- The demographic of pediatric HCC is evolving, with an increasing proportion of younger patients having congenital/metabolic liver diseases.
- Liver transplantation is the primary treatment for this cohort.
- Successful HBV immunization programs are likely contributing to this shift in pediatric HCC epidemiology.
Abstract:
HCC is the second most common malignant liver tumor of childhood. It typically affects children with a median age of 10-14 yr on background hepatitis B-related liver disease and is often metastatic or locally advanced at diagnosis. Children below the age of five yr typically constitute <10% of all children with HCC. In these children, it occurs on a background of congenital or metabolic liver disease. The records of all children with HCC who presented to our department over a six-yr study period were reviewed. Twelve patients with a median age of 5.9 yr (range 1.6-15.4) were diagnosed to have HCC. All patients underwent liver transplantation, and none were resected. Eleven patients had background congenital or metabolic liver disease. All five of those with hereditary tyrosinemia type 1 who presented to us were found to have HCC. No patient had hepatitis B-related liver (HBV) disease. Eight (66.7%) patients had incidentally discovered HCC on examination of the explant. Incidentally discovered HCC were smaller, well differentiated, and did not show microvascular invasion compared to those diagnosed preoperatively. There was no recurrence with a median follow-up of five months. The patient demographic for pediatric HCC is changing probably as a consequence of successful immunization against HBV. Younger patients with congenital and metabolic liver disease in whom liver transplantation is the ideal treatment are likely to constitute an ever-increasing proportion of patients with pediatric HCC as HBV disease is controlled or eradicated.
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